SCA10 is an autosomal dominant ataxia often accompanied with epilepsy caused by an expansion of a pentanucleotide (ATTCT) repeat in intron 9 of ATXN10. The disease is exclusively identified in Amerindian descents mainly in South America, in which they are likely to share a common ancestral origin [6, 7]. This evidence concerns the gene ATXN10 and epilepsy.